Congenital Conundrum: Unraveling the Puzzle of Double Outlet Right Ventricle for the Neonatologist.

Double outlet right ventricle (DORV) is a complex congenital heart disease with a wide spectrum of anatomical variations and clinical presentations. In this conotruncal anomaly, greater than 50% of the semilunar valve diameter of both the aorta and pulmonary artery arises from the morphologic right ventricle. Additionally, patients with DORV have a ventricular septal defect that allows blood to egress from the left ventricle and may obstruct either the pulmonary or systemic circulations. Physiological characteristics differ among patients based on the orientation of the great arteries, the presence of additional intracardiac shunts, and cardiac abnormalities. A thorough understanding of an individual's clinical features based on their DORV phenotype is essential for clinicians caring for these vulnerable patients. Knowledge of the expected postnatal hemodynamic derangements as transitional physiology evolves allows for tailored therapeutic approaches at the bedside. In this review, we will discuss the main DORV subtypes, delineate the physiology of each subtype, and provide clinical considerations and medical management strategies to guide the care for patients with this complex cardiac lesion.